Recurrent congenital haemangiopericytoma in a child

Abstract

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs.

Multifocal Congenital Hemangiopericytoma

“…HPC is a mesenchymal tumor that Stout and Murray first described in 1942 . Some authors believe that it originates from Zimmermann's vascular pericytes, which are contractile fusiform cells that surround capillaries and postcapillary venules , but the exact origin of the lesion is unclear. Given that the clinical and pathologic features overlap with those of myofibromatosis and solitary fibrous tumor (SFT), it has been suggested that these three entities are related variants of a continuous spectrum and that their histologic characteristics may correspond to different stages of maturation .…”

“…HPCs may be located anywhere, especially in the musculoskeletal system; the distal parts of the limbs are affected in 30% to 50% of diagnosed cases . There are reports of HPCs in the neck, mediastinum, armpit, arm, abdomen, thigh, and clitoris .…”

“…Complementary examinations are important for establishing the diagnosis by determining the extent of the tumor and planning treatment . Angiography shows the vascular pattern and MRI determines the size and extent of the tumor, as well as its effects on adjacent structures, which is helpful for presurgical planning .…”

“…Surgical excision is still the first choice of treatment , because it is associated with good prognosis and low relapse rate. Complete tumor excision and adjuvant chemotherapy reduce the risk of local recurrence . In our patient, because of the very large size of the tumor in the forearm, which would have required mutilating surgery and probable limb amputation, and the multifocal presentation, which would have hindered complete tumor removal, we chose chemotherapy before surgery, with excellent results.…”

Large Plantar Mass in a Newborn

“…There is no consensus on treatment for solitary infantile hemangiopericytomas, but radical surgical excision has been the standard of care in the past. Chemotherapy has also been used for recurrent lesions and for lesions that cause functional impairment and are not amenable to surgery (13). As many lesions resolve spontaneously with no intervention and there is little risk of metastasis, perhaps a more conservative approach should be adopted for solitary lesions (14).…”